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NCCN Flash Updates: NCCN Guidelines Updated for Pediatric Aggressive Mature B-Cell Lymphomas

NCCN has published updates to the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines^®), the NCCN Drugs & Biologics Compendium (NCCN Compendium^®), the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™) for Pediatric Aggressive Mature B-Cell Lymphomas. These NCCN Guidelines^® are currently available as Version 1.2025.

Link directly to the Updates section of the NCCN Guidelines: Pediatric Aggressive Mature B-Cell Lymphomas

PBCL-1

• Subtypes moved to PBCL-2.
• Additional diagnostic testing moved to header.
• New node: Post-Transplant Lymphoproliferative Disorders (PTLD) after solid organ transplant (SOT)
• Footnote c revised: Principles of Diagnostic Pathology (PBCL-A). See also the Use of Immunophenotyping/Genetic Testing in Differential Diagnosis of Mature B-Cell and NK/T-Cell Neoplasms (NHODG-A) in the NCCN Guidelines for B-Cell Lymphomas. (Also for subsequent pages)

PBCL-2

• Additional Diagnostic Testing
  • Useful Under Certain Circumstances
    • Bullet 4 revised: IHC/ISH Panel: MYC (IHC) and Kappa/Lambda (ISH)
• Footnote e revised: Typical immunophenotype of PMBL: sIg-, B-cell antigens+ (CD19+, CD20+, CD79a+, and PAX5+), CD23+, CD30+, MUM1+, BCL2+/-, and BCL6+/-. EBV-EBER is negative. See Use of Immunophenotyping/Genetic Testing in Differential Diagnosis of Mature B-Cell and NK/T-Cell Neoplasms (NHODG-A) in the NCCN Guidelines for B-Cell Lymphomas.
• Footnote i revised: Double- and triple-hit lymphomas (HGBL with MYC and BCL2 or BCL6 rearrangements) are currently not well described or studied in the pediatric population but FISH for BCL2 and BCL6 rearrangements may be considered in the AYA population. Pediatric HGBL is treated with the same regimens as pediatric BL.
• Footnote k revised: This is an uncommon variant of BL without MYC rearrangement but with 11q aberration (Campo E, et al. Blood 2022;140:1229-1253; Alaggio R, et al. Leukemia 2022;36:1720-1748 WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. [WHO classification of tumours series, 5th ed; vol 11]. Lyon [France]: International Agency for Research on Cancer; 2024). Optimum management of this rare subtype is undefined, although it is most often treated like typical BL.

PBCL-3

• Workup
  • Essential
    • Bullet 7, sub-bullet 1 revised: Cerebrospinal fluid (CSF) cell count and differential
    • Bullet 7, sub-bullet 2 revised: Cytology of CSF, including total nucleated cell count and morphologic review of cytospin
  • Useful Under Certain Circumstances
    • Bullet 1 revised: MRI of the head with and without contrast, if clinically indicated
    • Bullet 2 revised: MRI of the spine with and without contrast, if clinically indicated
    • Last bullet revised: Consider baseline immunoglobulin panel prior to using rituximab or if there are any underlying concerns about immunodeficiency
• Column 2, top pathway, bullets added:
  • LBCL with IRF4/MUM1 rearrangement
  • HGBL with 11q aberrations
  • HGBL with MYC and BCL2 or BCL6 rearrangements

PBCL-4

• Staging
  • Stage I revised: Single tumor (extranodal) or single anatomical area (nodal), with exclusion of mediastinum and abdomen A single tumor not in the mediastinum and abdomen

Burkitt Lymphoma and Diffuse Large B-Cell Lymphoma

PBCL-7

• Footnote ee revised: An FDA-approved biosimilar is an appropriate substitute for any recommended systemic biologic therapy in the NCCN Guidelines. An FDA-approved biosimilar is an appropriate substitute for rituximab. (Also for PBCL-8, 9, 11 and as footnote d on PMBL-1)

PBCL-10

• Late effects monitoring
  • Bullet 1 revised: Attention to cardiac, gonadal, and neurocognitive function, bone health, and risk of secondary leukemia. (See Children’s Oncology Group Survivorship Guidelines; see also the NCCN Guidelines for Survivorship) (Also for PMBL-1)
  • Bullet 2 added: Psychosocial support and counseling: See NCCN Guidelines for Distress Management. (Also for PMBL-1)

Primary Mediastinal Large B-Cell Lymphoma

PMBL-1

• Induction therapy/initial treatment
  • Added after clinical trial (preferred): In the absence of a clinical trial, suggested regimens include: (Also for PMBL-2)
  • Bullet 1 revised: Dose-adjusted-EPOCH-R (preferred) (6 cycles)
• Footnote e revised: Avoidance of RT is strongly preferred in pediatric patients to reduce the risk of late complications from normal tissue damage. There are not enough data on the use of RT in pediatric patients with PMBL. When RT is used, it should be delivered using advanced RT techniques to minimize dose to organ at risk (OAR) (heart, cardiac substructures, lungs, breast, spinal cord, etc). See Principles of Radiation Therapy in the NCCN Guidelines for Pediatric Hodgkin Lymphoma. Recommendations for normal tissue dose constraints can be found in Principles of Radiation Therapy in the NCCN Guidelines for Hodgkin Lymphoma. (Also for PMBL-2)

PMBL-2

• Treatment
  • Last bullet: Brentuximab vedotin + nivolumab was added as a category 2A recommendation.

PBCL-B 9 of 15

• Principles of Systemic Therapy
  • Header revised: Preferred Regimens for Induction Therapy (Also for PBCL-B 11 through 13)
  • Bullet 1 revised: Dose Adjusted-EPOCH-R (etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, rituximab) (preferred)

PBCL-B 10 of 15

• Bullet 5 revised: If ANC <1x 10⁹/l or platelets <100 x 10⁹/l on day 21, delay up to 1 week. Granulocyte colony-stimulating factor (G-CSF) (eg, pegfilgrastim) may be started for ANC <1x 10⁹/l and stopped 24 hours before treatment. If counts still low after 1-week delay, decrease 1 dose level below last course.

PBCL-B 14 of 15

• Footnote b added: An FDA-approved biosimilar is an appropriate substitute for any recommended systemic biologic therapy in the NCCN Guidelines.
• Footnote i revised: Avoidance of RT is strongly preferred in pediatric patients to reduce the risk of late complications from normal tissue damage. There are not enough data on the use of RT in pediatric patients with PMBL. When RT is used, it should be delivered using advanced RT techniques to minimize dose to OAR (heart, cardiac substructures, lungs, breast, spinal cord, etc). See Principles of Radiation Therapy in the NCCN Guidelines for Pediatric Hodgkin Lymphoma. Recommendations for normal tissue dose constraints can be found in Principles of Radiation Therapy in the NCCN Guidelines for Hodgkin Lymphoma.
• Footnote removed: An FDA-approved biosimilar is an appropriate substitute for rituximab.

PBCL-D 2 of 4

• Principles of Supportive Care
  • Mass Legions at Presentation
    • Bullet 1 revised: In pediatrics, there are multiple publications of spinal cord compression, massive kidney enlargement, intussusception, ovarian masses, chest masses, and facial masses. There may be increased risk of thrombosis due to mass lesions.

Pediatric Post-Transplant Lymphoproliferative Disorders

• New pages added for the diagnosis and management of pediatric post-transplant lymphoproliferative disorders (PTLD).

 

For the complete updated versions of the NCCN Guidelines, NCCN Guidelines with NCCN Evidence Blocks™, the NCCN Drugs & Biologics Compendium (NCCN Compendium^®), the NCCN Biomarkers Compendium^®, the NCCN Chemotherapy Order Templates (NCCN Templates^®), the NCCN Radiation Therapy Compendium™, and the NCCN Imaging Appropriate Use Criteria (NCCN Imaging AUC™), please visit NCCN.org.

To view the NCCN Guidelines for Patients^®, please visit NCCN.org/patientguidelines.

Free NCCN Guidelines apps for iPhone, iPad, and Android devices are now available! Visit NCCN.org/apps.

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